Strain‐ and age‐dependent loss of sarcoglycan complex in cardiomyopathic hamster hearts and its re‐expression by δ‐sarcoglycan gene transfer in vivo

Abstract
The δ‐sarcoglycan (SG) gene is deleted in hamsters with hereditary cardiomyopathies. Immunological analyses of heart before, but not after, the progression of cardiomyopathy (CM) revealed that the BIO 14.6 strain, a model of hypertrophic CM, heterogeneously preserved α‐ and γ‐SG with loss of β‐ and δ‐SG. In contrast, the TO‐2 strain, a model of dilated CM, did not show either SG. Furthermore, in vivo transfer of the full length δ‐SG gene to TO‐2 hearts expressed all four SGs. Thus, this age‐ and strain‐dependent features suggest a more feasible setting for TO‐2 than BIO 14.6 to verify both CM progression and the efficacy of gene therapy.

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